National Organization for Rare Disorders, Inc.

Papillitis

Important
It is possible that the main title of the report Papillitis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Optic Nerve Papillitis

Disorder Subdivisions

  • None

Related Disorders List

Information on the following diseases can be found in the Related Disorders section of this report:

  • Giant Cell Arteritis
  • Multiple Sclerosis
  • Retrobulbar Neuritis

General Discussion

Papillitis, also known as optic neuritis, is characterized by inflammation and deterioration of the portion of the optic nerve known as the optic disk. Also referred to as the "blind spot," the optic disk (optic papilla) is that portion of the optic nerve that enters the eye and joins with the nerve-rich membrane lining the eye (retina). The optic nerves are the pair of nerves (second cranial nerves) that transmit impulses from the retina to the brain. Individuals with papillitis experience loss of vision in one eye that may occur within several hours of onset. The severity of visual impairment may vary from case to case, ranging from slight visual deficiency to complete loss of light perception. In addition, affected individuals experience a reduction in color perception. In some cases, spontaneous recovery may occur. However, in other cases, permanent visual impairment may result if the underlying cause is not detected or treated. Papillitis may occur for unknown reasons, after a viral illness, or due to or in association with a number of different underlying disorders or other factors.

Symptoms

The symptoms of papillitis include loss of vision, pain in the eye, and interference with accurate color vision (dyschromatopsia).

Individuals with papillitis usually experience unilateral loss of vision. That is, they lose sight in one eye (about 70% of cases), usually within a short time (a few hours) of having become aware of diminished sight. This condition is usually rapidly progressive.

The intensity of vision impairment varies from case to case, ranging from slight visual deficiency to complete loss of light perception. In addition, affected individuals experience a reduction in color perception. In some cases, spontaneous recovery may occur. However, in other cases, permanent visual impairment may result if the underlying cause is not detected or treated. Papillitis may occur for unknown reasons, after a viral illness, or due to or in association with a number of different underlying disorders or other factors.

Causes

There are many possible causes of papillitis. These include diseases that result in damage to the lining of nerves (demyelinating diseases) such as multiple sclerosis and encephalomyelitis; viral or bacterial infections such as polio, measles, pneumonia, or meningitis; nutritional or metabolic disorders such as diabetes, pernicious anemia, and hyperthyroidism; secondary complications of other diseases; reactions to toxic substances such as methanol, quinine, salicylates, and arsenic; and trauma.

In patients over 60 years of age, a common cause of papillitis is temporal arteritis (giant cell arteritis). In such cases, papillitis can spread to the other eye resulting in bilateral blindness.

Affected Populations

Papillitis affects males and females in equal numbers and can occur at any age. A percentage of people with papillitis may eventually be diagnosed with multiple sclerosis. (for more information on this disorder, see the Related Disorders section of this report.)

Related Disorders

The following disorders may be associated with papillitis.

Giant cell arteritis is a chronic inflammatory disease of the branches of the aortic arch. This disorder is found principally in the temporal and occipital arteries, but may develop in almost any of the large arteries. It rarely involves veins. Papillitis may occur in people with giant cell arteritis. (For more information on this disorder, choose "Arteritis, Giant Cell" as your search term in the Rare Disease Database.)

Multiple sclerosis is a chronic disease of the brain and spinal cord (central nervous system) which may be progressive, relapsing and remitting, or stable. People with MS have small nerve lesions called plaques that may form randomly throughout the brain and spinal cord. These patches prevent proper transmission of nerve signals and thus result in a variety of neurological symptoms. (For more information on this disorder, choose "Multiple Sclerosis" as your search term in the Rare Disease Database.)

Retrobulbar neuritis is an inflammation of that portion of the optic nerve that lies behind the eyeball. Many cases of this disease are caused by multiple sclerosis while others may be due to viral or infectious disorders. In most cases there may be no apparent cause. This disease usually affects one eye and is characterized by pain associated with movement of the eye, headache and a rapid and progressive loss of vision.

Standard Therapies

Diagnosis
Diagnostic testing may include testing for visual acuity, testing for color vision, examination of the optic disc by means of ophthalmoscopy and magnetic resonance imaging.

Treatment
If spontaneous remission does not occur in people with papillitis it is usually treated with the corticosteroid drugs prednisone or methylprednisolone. Other treatment is symptomatic and supportive.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.

For information about clinical trials being conducted at the National Institutes of Health (NIH) Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com


MidAmerica Neuroscience Institute is currently recruiting patients experiencing a first "demyelinating event" for a clinical trial. For information, go to www.clinicaltrial.gov or contact:

Laurie A. Dressman, RN, BA at 816-753-8800 ext 124, at
MidAmerica Neuroscience Institute
Kansas City, MO 64108

References

Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:739.

Bennett JC, Plum F., eds. Cecil Textbook of Medicine. 20th ed. W.B. Saunders Co., Philadelphia, PA; 1996:2016-17.

Stein JH, Hutton JJ, Kohler PO, et al., eds. Internal Medicine. 4th ed. Mosby-Yearbook, Inc., St. Louis, MO. 1994:1152-53.

Kanski JJ., ed. Clinical Ophthalmology. 4th ed. Butterworth-Heinemann. Oxford, UK; 1999:590-93.

Newell FW., ed. Ophthalmology: Principles and Concepts. 7th ed. Mosby Year Book, St. Louis, MO; 1991:348-49.

REVIEW ARTICLES
Myers TD, Smith JR, Wertheim MS, et al. Use of corticoid sparing systemic immunosuppression for treatment of corticoid dependent optic neuritis not associated with demyelinating disease. Br J Ophthalmol. 2004;17:3-8.

Margalit E, Sadda SR. Retinal and optic nerve diseases. Artif Organs. 2003;27:963-74.

Parisi V. Correlations between morphological and functional retinal impairment in patients affected by ocular hypertension, glaucoma, demyelinating optic neuritis and Alzheimer's disease. Semin Ophthalmol. 2003;18:50-57.

Kesler A, Pianka P. Toxic optic neuropathy. Curr Neurol Neurosci Rep. 2003;3:410-14.

Chan JW. Optic neuritis in multiple sclerosis. Ocul Immunol Inflamm. 2002;10:161-86.

JOURNAL ARTICLES
Pirko I, Blauwet LK, Lesnick TJ, et al. The natural history of recurrent optic neuritis. Arch Neurol. 2004;61:1401-05

[No authors listed]. Neurologic Impairment 10 Years after Optic Neuritis. Arch Neurol. 2004;61:1386-89.

Hickman SJ, Toosy AT, Miszkiel KA, et al. Visual recovery following acute optic neuritis: A clinical, electrophysiological and magnetic resonance imaging study. J Neurol. 2004;251:996-1005.

Lim ET, Grant D, Pashenkov M, et al. Cerebrospinal fluid levels of brain specific proteins in optic neuritis. Mult Scler. 2004;10:261-65.

Craenen G, Brown SM, Freedman KA, et al. Rapid, painless unilateral vision loss in a 37-year-old healthy woman. Surv Ophthalmol. 2004;49:343-48.

Beck RW, Gal RL, Bhatti MT, et al. Visual function more than 10years after optic neuritis: experience of the optic neuritis treatment trial. Am J Ophthalmol. 2004;137:77-83. Errata in: Am J Ophthalmol. 2004;137:following 793. Am J Ophthalmol. 2004;138:following 321.

Fazzone HE, Lefton DR, Kupersmith MJ. Optic neuritis: Correlation of pain and magnetic resonance imaging. Ophthalmology. 2003;110:1646-49.

FROM THE INTERNET
Swallow C. Optic Neuritis. emedicine. Last Updated: July 20, 2004. 9pp.
www.emedicine.com/radio/topic488.htm

Giovannini J, Chrousos G. Pseudopapilledema. emedicine. Last Updated: August 26, 2002. 6pp.
www.emedicine.com/oph/topic615.htm

Papillitis. The Merck Manual. 2004. 2pp.
www.merck.com/mrkshared/mmanual/section8/chapter101/101b.jsp

Resources

National Association for Visually Handicapped
22 West 21st Street
New York, NY 10010
USA
Tel: (212)889-3141
Fax: (212)727-2931
Email: staff@navh.org
Internet: http://www.navh.org

NIH/National Eye Institute
Building 31 Rm 6A32
31 Center Dr MSC 2510
Bethesda, MD 20892-2510
United States
Tel: (301)496-5248
Fax: (301)402-1065
Email: 2020@nei.nih.gov
Internet: http://www.nei.nih.gov/

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
TDD: (888)205-3223
Email: ordr@od.nih.gov
Internet: http://rarediseases.info.nih.gov/Default.aspx

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  3/30/2008
Copyright  1989, 1994, 1997, 2005 National Organization for Rare Disorders, Inc.


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Topic Contents
 Synonyms
 Disorder Subdivisions
 Related Disorders List
 General Discussion
 Symptoms
 Causes
 Affected Populations
 Related Disorders
 Standard Therapies
 Investigational Therapies
 References
 Resources